Hemorrhagic diatheses in children. Gastrointestinal

bleedings.

Sakharova I. Ye., MD, PhD

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The stopping of a bleeding is carried out due to three parts of a

hemostasis:

1.1. Vascular integrity.Vascular integrity.

2.2. Qualitative and quantitative Qualitative and quantitative characteristics of platelets. characteristics of platelets.

3.3. Presence of coagulation factors in Presence of coagulation factors in blood.blood.

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According to this all hemorrhagic diatheses are divided into 3

groups:

1. Vasopathies 2. Thrombopathias 3. Coagulopathies

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Schönlein-Henoch purpura (synonims - anaphylactoid purpura, allergic angiitis, small-vessel vasculitis, hemorrhagic vasculitis, Henoch-Schönlein disease) is one of the collagen vascular diseases in which basis lays immune complex mechanism of small vessels wall damaging with skin, joints, intestine and kidneys affection.

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Clinical features.

Papular- macular hemorrhagic purpuric lesions. Typical places of rush localization: extensor surfaces of legs, on the feet, over the joints, on the buttocks; occasionally, they may occur on the hands, extensor surfaces of arms, elbows, and face, but very rarely on the trunk.

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Clinical features of the Schönlein-Henoch purpura.

joints involvement acute abdominal pain, vomiting,

melena renal involvements (microscopic

hematuria, with or without proteinuria)

scrotal involvement (epydidimitis, orchitis, and scrotal bleeding)

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C ongenital(Fanconi pancytopenia,

Wiskott-Aldrich syndrome)

Thrombocytessynthesis

disturbances

H eteroim m uneA utoim m uneIzoim m une

Im m une N onim m une(sepsis, D IC -

syndrom , H U S)

Increaseof p leteletsdestruction

D isturbancesof p lateletsdistribution

A cquired

Thrombocytopenies

C ongenital(Glanzmann thromboasthenia

Bernard-Soulier syndrom,Von Willebrand disease)

A cquired

Thrombocytopathias

Platelet defects

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Idiopathic thrombocytopenic purpura (ITP, primary immune thrombocytopenic purpura, autoimmune thrombocytopenic purpura)

describes an autoimmune disorder in which the number of circulating platelets is less than 150 G/l.

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Clinical features.

• The onset of the disease is usually sudden. The symptoms of intoxication and fever usually are absent.

• Skin purpura, which arises either spontaneously or secondary to trauma. The type of rush is petechial-bruise.

• The second frequent clinical sign are bleedings. In the beginning of the disease can be nose bleeding (epistaxis), bleeding from gums, mucous membranes, gastrointestinal tract, kidneys and metrorrhagias (uterinal bleedings).

• Hemorrhage into the central nervous system, the most serious complication of thrombocytopenia.

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Hemophilia A and B are inherited bleeding disorders caused by deficiencies of clotting factor VIII (F VIII - antigemophilic globulin (AHG)) and factor IX (F IX - plasma thromboplastin component (PTC) or Christmas factor) correspondingly.

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Mechanism of hemophilia inheritance

Xy xx

xy Xx xy xyXx

Xy xy xx Xx Xy

XX Xx xy Xy

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Prophylactic treatment

The aim of this treatment is to maintain 5 % factor activity in patient’s blood.

Start from the age of 1-2 years.

Use monoclonal-antibody purified F VІІІ , F ІХ and recombinant F VІІІ , F ІХ 3 times in week in hemophilia A and 2 times in week in hemophilia B 25-40 IU/kg.

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Treatment of acute bleeding episodes

Hemophilia A– Fresh frozen plasma (100 ml=80IU АHG)

Dose is 10-15 ml/kg IV during 30-60 min, repeat after 8-12 hours.

– Cryoprecipitate – Monoclonal-antibody purified F VІІІ and

recombinant F VІІІ

Hemophilia B– Fresh frozen plasma – Monoclonal-antibody purified F IX and

recombinant F IX

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During severe or dangerous (e.g. CNS, retroperitoneal) bleeds need to obtain 50-100% factor activity for 7-10 days. For less critical situations (e.g. dental extractions, haematuria, soft tissue bleeds), 20-50% factor activity for 2-7 days are generally sufficient. For uncomplicated haemarthroses or superficial muscle or soft tissue bleeds, 20-30% for 1-2 days.

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Laboratory differential diagnostics of hemorrhagic diatheses

 Count of platelets

Time of bleeding

Coagulation time

Clot retrac-tion

Coagulogram

Hem. vaskul.

ITP

Trombopathia

Hemophilia

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Success is not final, failure is not fatal. It is the courage tocontinue that counts.

Winston Churchill

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